Etiology and Initial Management of Short Bowel Syndrome

Abstract

The normal human small intestine ranges between 3 and 8 m in length, depending on whether measurement is made by radiologic or surgical techniques or at autopsy when the intestine may be desicated.1Crenn P. Haniche M. Valleur P. et al.Surgical versus radiological evaluation of remaining small bowel length in short bowel syndrome.Gastroenterology. 1996; 110 (abstr): A321Abstract Full Text PDF PubMed Scopus (4) Google Scholar, 2Bryant J. Observations upon the growth and length of the human intestine.Am J Med Sci. 1924; 167: 499-520Crossref Google Scholar, 3Slater G. Aufses Jr, A.H. Small-bowel length in Crohn’s disease.Am J Gastroenterol. 1991; 8: 1037-1040Google Scholar, 4Fanucci A. Cerro P. Fraracci L. Letto F. Small-bowel length measured by radiology.Gastrointest Radiol. 1984; 9: 349-351Crossref PubMed Scopus (34) Google Scholar, 5Nightingale J.M.D. Bartram C.I. Lennard-Jones J.E. Length of residual small bowel after partial resection correlation between radiographic and surgical measurements.Gastrointest Radiol. 1991; 16: 305-306Crossref PubMed Scopus (81) Google Scholar Short bowel syndrome is defined in adults as <200 cm of small intestine. Although usually acquired because of one or more enterectomies, short bowel syndrome may also be congenitally acquired. Nutrient, electrolyte, and fluid absorption is proportional to the amount of residual intestine. The degree of intestinal function is better described in terms of energy absorption/loss rather than length of residual intestine.6Jeppesen P.B. Mortensen P.B. Intestinal failure determined by measurements of intestinal energy and wet weight absorption.Gut. 2000; 46: 701-707Crossref PubMed Scopus (134) Google Scholar Intestinal failure may be defined as the inability to sustain adequate nutritional, electrolyte, or hydrational status in the absence of specialized nutritional support. This requires an increase in oral intake because absorption is compromised. Clinically, nutrient assimilation is a function of intake and absorption. As such, some patients with short bowel syndrome will not have sufficient loss of functional capacity as to develop intestinal failure. Significant individual variability in jejunal absorption efficiency may be encountered.7Florent C. Flourie B. Leblond A. et al.Influence of chronic lactulose ingestion on the colonic metabolism of lactulose in man (an in vivo study).J Clin Invest. 1985; 75: 608-613Crossref PubMed Scopus (214) Google Scholar The patients with the greatest risk for development of dehydration, generalized protein-calorie malnutrition, and multiple nutrient deficiencies are those with a duodenostomy or jejunoileal anastomosis and <35 cm of residual small intestine; jejunocolic or ileocoloic anastomosis, and <60 cm of residual small intestine; or end jejunostomy with <115 cm of residual small intestine.6Jeppesen P.B. Mortensen P.B. Intestinal failure determined by measurements of intestinal energy and wet weight absorption.Gut. 2000; 46: 701-707Crossref PubMed Scopus (134) Google Scholar, 8Messing B. Crenn P. Beau P. et al.Long-term survival and parenteral nutrition dependence in adult patients with short bowel syndrome.Gastroenterology. 1999; 117: 1043-1050Abstract Full Text Full Text PDF PubMed Scopus (487) Google Scholar, 9Carbonnel F. Cosnes J. Chevret S. et al.The role of anatomic factors in nutritional autonomy after extensive small bowel resection.JPEN J Parenter Enteral Nutr. 1996; 20: 275-280Crossref PubMed Scopus (205) Google Scholar, 10Nightingale J.M.D. Lennard-Jones J.E. Gertner D.J. et al.Colonic preservation reduces need for parenteral therapy, increases incidence of renal stones, but does not change high prevalence of gallstones in patients with a short bowel.Gut. 1992; 33: 1493-1497Crossref PubMed Scopus (252) Google Scholar, 11Nordgaard I. Hansen B.S. Mortensen P.B. Importance of colonic support for energy absorption as small-bowel failure proceeds.Am J Clin Nutr. 1996; 64: 222-231PubMed Google Scholar Those patients with residual colon in continuity will have enhanced energy and fluid absorption. Hence, such patients can tolerate greater loss of small intestine and retain their nutritional autonomy. It is unclear how many individuals in the United States suffer from short bowel syndrome, but, based on the numbers in Europe, the incidence is probably approximately 2 individuals per million.12Mughal M. Irving M. Home parenteral nutrition in the United Kingdom and Ireland.Lancet. 1986; 200: 383-386Abstract Scopus (140) Google Scholar More recent data from 1993 indicated that the incidence and prevalence of home parenteral nutrition, for which short bowel syndrome was the most prevalent indication, had increased slightly to 2 or 3 per year per million inhabitants and 4 per year per million, respectively.13Van Gossum A. Espen-Han GroupHome parenteral nutrition (HPN) in adults a multicentre survey in Europe in 1993.Clin Nutr. 1996; 15: 53-58Abstract Full Text PDF PubMed Scopus (116) Google Scholar, 14Van Gossum A. Colomb V. Hebuterne X. et al.Home parenteral nutrition (HPN) in children a multicentre study in Europe in 1997.Clin Nutr. 1998; 17 (abstr): 42Abstract Full Text PDF Google Scholar The most recent European survey, in 1997, indicated that the incidence of home total parenteral nutrition (TPN) had remained approximately 3 per million and that the prevalence had increased to 4 per million.15Van Gossum A. Bakker H. Bozetti F. et al.Home parenteral nutrition in adults a European multicentre study in 1997.Clin Nutr. 1999; 18: 135-140Abstract Full Text PDF PubMed Scopus (238) Google Scholar The largest single group of patients who received home TPN were those with short bowel syndrome (35%). In comparison, the most recent data for incidence and prevalence in the United States is from 1992. At that time, it was estimated based on extrapolated data from the Oley Foundation Home TPN Registry that approximately 40,000 patients required TPN each year.16Oley Foundation. North American home parenteral and enteral nutrition patient registry annual report, 1994.Google Scholar Approximately 26% of the patients in the Oley Registry had short bowel syndrome, although some patients with a primary TPN indication of malignancy or radiation enteritis may have had short bowel syndrome as well. Patients with short bowel syndrome who did not require home TPN or were successfully weaned from home TPN are not reflected in these statistics. Approximately half of the short bowel patients who initially require TPN can be weaned off of TPN successfully in optimal settings.8Messing B. Crenn P. Beau P. et al.Long-term survival and parenteral nutrition dependence in adult patients with short bowel syndrome.Gastroenterology. 1999; 117: 1043-1050Abstract Full Text Full Text PDF PubMed Scopus (487) Google Scholar Therefore, the number of patients with short bowel syndrome may be substantially greater than previously estimated. A registry of short bowel patients, including those who require TPN permanently, transiently, and not at all, should be implemented. Short bowel syndrome may be a congenital or acquired condition. Infants may be born with congenital jejunal or ileal atresia. Otherwise, short bowel syndrome results from surgical resection of bowel. This is usually related to multiple resections for recurrent Crohn’s disease, massive enterectomy made necessary because of a catastrophic vascular event such as mesenteric arterial embolism, venous thrombosis, volvulus, trauma, or tumor resection in adults, and, in children, gastroschisis, necrotizing enterocolitis (NEC), volvulus, and extensive aganglionosis. Functional short bowel syndrome may also occur in cases of severe malabsorption in which the bowel length is often intact. Such conditions may include chronic intestinal pseudo-obstruction syndrome, refractory sprue, radiation enteritis, and congenital villus atrophy. Severe nutrient and fluid malabsorption occurs following extensive small intestinal resection. Patients with less than 100 cm of jejunum remaining generally have a net secretory response to food.17Nightingale J.M. Lennard-Jones J.E. Walker E.R. Farthing M.J. Jejunal efflux in short bowel syndrome.Lancet. 1990; 336: 765-768Abstract PubMed Scopus (163) Google Scholar Patients can be grouped into 2 distinct subgroups: those with intact colon in continuity and those without colon in continuity. The colon becomes an important digestive organ in patients with short bowel syndrome. Sodium, water, and some amino acids are absorbed in the colon9Carbonnel F. Cosnes J. Chevret S. et al.The role of anatomic factors in nutritional autonomy after extensive small bowel resection.JPEN J Parenter Enteral Nutr. 1996; 20: 275-280Crossref PubMed Scopus (205) Google Scholar, 18Woolf G.M. Miller C. Kurian R. Jeejeebhoy K.N. Nutritional absorption in short bowel syndrome.Dig Dis Sci. 1987; 32: 8-15Crossref PubMed Scopus (108) Google Scholar, 19Langefoged K. Olgaard K. Fluid and electrolyte absorption and renin-angiotensin-aldosterone axis in patients with severe short-bowel syndrome.Gastroenterology. 1979; 14: 729-735Google Scholar, 20Zibrik L. Dyer J. Ellis T. Shirazi-Beechey S.P. Amino acid transport in human colon in short bowel patients.Gastroenterology. 2003; 124 (abstr): A31Abstract Full Text PDF Google Scholar as well as energy from absorbed short-chain fatty acids. The intestine adapts as well to ensure more efficient absorption per unit length. Following massive enterectomy, the intestine hypertrophies, and nutrient absorption becomes more efficient. Although there are limited human data, observations in patients with massive enterectomy as well as in the functional short bowel patient (JI-bypass) indicate that the intestine lengthens some, but more importantly, diameter and villus height increase with a resultant increase in absorptive surface.21Solhaug J.H. Tvete S. Adaptive changes in the small intestine following bypass operation for obesity.Scand J Gastroenterol. 1978; 13: 401-408Crossref PubMed Scopus (43) Google Scholar, 22Doldi S.B. Intestinal adaptation following jejuno-ileal bypass.Clin Nutr. 1991; 10: 138-145Abstract Full Text PDF PubMed Scopus (37) Google Scholar, 23Dowling R.H. Booth C.C. Functional compensation after small-bowel resection in man.Lancet. 1966; ii: 146-147Google Scholar, 24Weinstein L.D. Shoemaker C.P. Hersh T. Wright H.K. Enhanced intestinal absorption after small bowel resection in man.Arch Surg. 1969; 99: 560-561Crossref PubMed Scopus (96) Google Scholar This process may evolve over 1 or 2 years.6Jeppesen P.B. Mortensen P.B. Intestinal failure determined by measurements of intestinal energy and wet weight absorption.Gut. 2000; 46: 701-707Crossref PubMed Scopus (134) Google Scholar, 9Carbonnel F. Cosnes J. Chevret S. et al.The role of anatomic factors in nutritional autonomy after extensive small bowel resection.JPEN J Parenter Enteral Nutr. 1996; 20: 275-280Crossref PubMed Scopus (205) Google Scholar, 25Kurkchubasche A.G. Rowe M.I. Smith S.D. Adaptation in short-bowel syndrome reassessing old limits.J Pediatr Surg. 1993; 28: 1069-1071Abstract Full Text PDF PubMed Scopus (82) Google Scholar Patients may be taught to adapt to their decreased absorption as well by dramatically increasing their food intake (hyperphagia). The presence or absence of the colon and ileocecal valve; location (jejunum versus ileum), health, and length of residual bowel; mucosal blood flow; patient age; and comorbid conditions such as Crohn’s disease, radiation enteritis, carcinoma, or pseudo-obstruction are important determinants in the functional adaptation process and clinical outcome.6Jeppesen P.B. Mortensen P.B. Intestinal failure determined by measurements of intestinal energy and wet weight absorption.Gut. 2000; 46: 701-707Crossref PubMed Scopus (134) Google Scholar, 26Carbonnel F. Cosnes J. Chevret S. et al.The role of anatomic factors in nutritional autonomy after extensive small bowel resection.JPEN J Parenter Enteral Nutr. 1996; 20: 275-280Crossref PubMed Google Scholar, 27Kaufman S.S. Loseke C.A. Lupo J.V. et al.Factors affecting duration of parenteral hyperalimentation in children with short bowel syndrome.Gastroenterology. 1996; 110 (abstr): A809Abstract Full Text Full Text PDF PubMed Scopus (35) Google Scholar Although the length of remaining bowel necessary to prevent dependence on TPN is approximately 100 cm in the absence of an intact and functional colon, or 60 cm in the presence of a completely functional colon,6Jeppesen P.B. Mortensen P.B. Intestinal failure determined by measurements of intestinal energy and wet weight absorption.Gut. 2000; 46: 701-707Crossref PubMed Scopus (134) Google Scholar, 9Carbonnel F. Cosnes J. Chevret S. et al.The role of anatomic factors in nutritional autonomy after extensive small bowel resection.JPEN J Parenter Enteral Nutr. 1996; 20: 275-280Crossref PubMed Scopus (205) Google Scholar, 28Borgstrom B. Dahlqvist A. Lundh G. Sjovall J. Studies of intestinal digestion and absorption in the human.JCI. 1957; 36: 1521-1536Crossref PubMed Scopus (515) Google Scholar the degree of adaptation and TPN dependence may be highly individualized. Adaptation to full enteral nutrition has been reported with as little as 10 cm of residual intestine in infants.25Kurkchubasche A.G. Rowe M.I. Smith S.D. Adaptation in short-bowel syndrome reassessing old limits.J Pediatr Surg. 1993; 28: 1069-1071Abstract Full Text PDF PubMed Scopus (82) Google Scholar Patients with a jejunostomy are at increased risk for TPN dependence, and those with a jejunal-ileal anastomosis are less likely to be TPN dependent. Animal models have suggested that enteroglucagon, glucagon peptide II, epidermal growth factor, growth hormone, cholecystokinin, gastrin, insulin, and neurotensin are involved in postresection intestinal adaptation.29Cisler J.J. Buchman A.L. Intestinal adaptation in short bowel syndrome.J Investig Med. 2005; 53: 402-413Crossref PubMed Scopus (58) Google Scholar There are little data on the role of either increased endogenous hormonal release or exogenous hormone supplementation during the intestinal adaptation phase in humans. Residual ileum is able to adapt and to assume the role of macronutrient absorption when jejunum has been resected. However, the specialized cells of the terminal ileum in which vitamin B-12/intrinsic factor receptors are located and in which bile salts are reabsorbed cannot be replaced by jejunal hypertrophy. The goal of medical therapy is for the patient to resume work and a normal lifestyle, or as normal of one as possible. This is undertaken via the use of specific measures to decrease gradually the requirement for TPN and, at best, to eliminate its need. The most important aspects of the medical management of the patient with short bowel syndrome are to provide adequate nutrition, including both macro- and micronutrients, to prevent energy malnutrition and specific nutrient deficiencies, to provide sufficient fluid to prevent dehydration, and to correct and prevent acid-base disturbances. Most macronutrients, including carbohydrate, nitrogen, and fat, are absorbed within the first 100 cm and up to 150 cm of jejunum.30Glynn C.C. Greene G.W. Winkler M.F. Albina J.E. Predictive versus measured energy expenditure using limits-of-agreement analysis in hospitalized, obese patients.JPEN J Parenter Enteral Nutr. 1999; 23: 47-154Crossref PubMed Scopus (74) Google Scholar Typically, patients who have undergone massive enterectomy require TPN for the first 7–10 days. The immediate goals during this period are survival and hemodynamic stability. Nutritional therapy should not be introduced until the patient is hemodynamically stable and fluid management issues are relatively stable. Patients should be provided approximately 25–35 kcal/kg/day and 1.0–1.5 kg/day of protein. It remains controversial whether intake should be based on actual body weight or ideal body weight.31Creen P. Morin M.C. July F. Penven S. Thuillier F. Messing B. Net digestive absorption and adaptive hyperphagia in adult short bowel patients.Gut. 2004; 53: 1279-1286Crossref PubMed Scopus (109) Google Scholar Enteral nutrition should be started as soon as possible once hemodynamic stability has been achieved; standard enteral formula is recommended. Enteral nutrition should be instituted gradually as tolerated. Once patients are able to eat, they should be encouraged to eat a regular diet but modified as described below. Patients should also be encouraged to adapt their diet to eat substantially more than what was usual prior to their catastrophic event (hyperphagia).32Ameen V.Z. Powell G.K. Jones L.A. Qualitation of fecal carbohydrate excretion in patients with short bowel syndrome.Gastroenterology. 1987; 92: 493-500PubMed Google Scholar Separation of liquids and solids has no effect on macronutrient, electrolyte, or mineral absorption or fecal volume or fecal weight.19Langefoged K. Olgaard K. Fluid and electrolyte absorption and renin-angiotensin-aldosterone axis in patients with severe short-bowel syndrome.Gastroenterology. 1979; 14: 729-735Google Scholar Dietary protein is first digested by pancreatic, gastric, and intestinal proteases and then absorbed as di- and tripeptides. Therefore, it was reasoned that if dietary protein were provided in a predigested form, it would be more readily absorbed. However, absorption of nitrogeneous macronutrients (or proteins) is least affected by the decreased intestinal absorptive surface in patients with short bowel syndrome. Therefore, the utility of peptide-based diets in such patients is theoretically without merit. McIntyre et al fed 7 patients with an end-jejunostomy (60–150 cm residual small bowel) a peptide-based or an essentially isocaloric and isonitrogenous polymeric formula. Energy, carbohydrate, nitrogen, fat, electrolyte, fluid, and mineral absorption and stool weight were similar regardless of the enteral formula provided.33McIntyre P.B. Fitchew M. Lennard-Jones J.E. Patients with a high jejunostomy do not need a special diet.Gastroenterology. 1986; 91: 25-33PubMed Google Scholar Uncontrolled data from Levy et al support these findings.34Levy E. Frileux P. Sandrucci S. et al.Continuous enteral nutrition during the early adaptive stage of the short bowel syndrome.Br J Surg. 1988; 75: 549-553Crossref PubMed Scopus (117) Google Scholar A small study of 6 patients (90–150 cm of residual jejunum and end-jejunostomy) reported by Cosnes et al, however, suggested that nitrogen absorption may be modestly improved with the use of a peptide-based diet, although similar to previous studies, energy, other macronutrient, electrolyte, mineral, and fluid absorption were unaffected.35Cosnes J. Evard D. Beaugerie L. et al.Improvement in protein absorption with a small peptide-base diet in patients with high jejunostomy.Nutrition. 1992; 8: 406-411PubMed Google Scholar These studies were all very small, the study populations somewhat heterogeneous, and the various peptides used in the formulas differed significantly, as did the type and amount of fat (long-chain triglycerides vs medium-chain triglycerides). It is therefore difficult to make definitive comparisons among studies. The amino acid glutamine, together with glucose, is the preferred fuel for the small intestinal enterocyte.36Windmueller H.G. Spaeth A.E. Identification of ketone bodies and glutamine as the major respiratory fuels in vivo for postabsorptive rat small intestine.J Biol Chem. 1978; 253: 69-76Abstract Full Text PDF PubMed Google Scholar Rodent TPN models suggested that either parenteral or enteral glutamine supplements could effect more rapid and more significant bowel adaptation following massive enterectomy.37Guarino A. Canani R.B. Iafusco M. et al.In vivo and in vitro effects of human growth hormone on rat intestine ion transport.Pediatr Res. 1995; 37: 576-580Crossref PubMed Scopus (25) Google Scholar, 38Souba W. Klimberg S. Plumley D. et al.The role of glutamine in maintaining a healthy gut and supporting the metabolic response to injury and infection.J Surg Res. 1990; 48: 383-391Abstract Full Text PDF PubMed Scopus (308) Google Scholar Therefore, it was thought that glutamine supplementation in humans would have a similar effect. Although an early case series of 10 patients who were beyond the usual 1–2 year adaptation period suggested that glutamine, combined with growth hormone supplementation, and a high complex carbohydrate diet could result in decreased stool output and increased absorption of energy, protein, carbohydrate, sodium, and water,39Byrne T.A. Morrissey T.B. Nattakom T.V. et al.Growth hormone, glutamine, and a modified diet enhance nutrient absorption in patients with severe short bowel syndrome.Jpen. 1995; 19: 296-302Crossref PubMed Scopus (233) Google Scholar 2 subsequent, double-blinded, randomized placebo-controlled trials failed to confirm any of these effects.40Scolapio J.S. Camilleri M. Fleming C.R. et al.Effect of growth hormone, glutamine, and diet on adaptation in short-bowel syndrome a randomized, controlled trial.Gastroenterology. 1997; 113: 1074-1081Abstract Full Text Full Text PDF PubMed Scopus (260) Google Scholar, 41Beaugerie L. Carbonnel F. Hecketsweiler B. et al.Effects of an isotonic oral rehydration solution, enriched with glutamine, on fluid and sodium absorption in patients with a short-bowel.Aliment Pharmacol Ther. 1997; 11: 741-746Crossref PubMed Scopus (32) Google Scholar In addition, Scolapio et al showed that glutamine and growth hormone supplementation did not lead to morphologic changes in the intestine.40Scolapio J.S. Camilleri M. Fleming C.R. et al.Effect of growth hormone, glutamine, and diet on adaptation in short-bowel syndrome a randomized, controlled trial.Gastroenterology. 1997; 113: 1074-1081Abstract Full Text Full Text PDF PubMed Scopus (260) Google Scholar Glutamine-supplemented oral rehydration solution (see fluid and electrolyte management) was associated with decreased Na absorption and a trend toward decreased fluid absorption in a small controlled trial in 6 patients.41Beaugerie L. Carbonnel F. Hecketsweiler B. et al.Effects of an isotonic oral rehydration solution, enriched with glutamine, on fluid and sodium absorption in patients with a short-bowel.Aliment Pharmacol Ther. 1997; 11: 741-746Crossref PubMed Scopus (32) Google Scholar All of the patients studied by Byrne et al39Byrne T.A. Morrissey T.B. Nattakom T.V. et al.Growth hormone, glutamine, and a modified diet enhance nutrient absorption in patients with severe short bowel syndrome.Jpen. 1995; 19: 296-302Crossref PubMed Scopus (233) Google Scholar had colon in continuity; it is likely the treatment-associated increase in energy absorption was related solely to the increased complex carbohydrate diet as discussed above. Treatment with growth hormone and glutamine in the setting of short bowel syndrome has been associated with significantly increased extracellular fluid and peripheral edema.40Scolapio J.S. Camilleri M. Fleming C.R. et al.Effect of growth hormone, glutamine, and diet on adaptation in short-bowel syndrome a randomized, controlled trial.Gastroenterology. 1997; 113: 1074-1081Abstract Full Text Full Text PDF PubMed Scopus (260) Google Scholar, 42Szkudlarek J. Jeppesen P.B. Mortensen P.B. Effect of high dose growth hormone with glutamine and no change in diet on intestinal absorption in short bowel patients a randomised, double-blind, crossover, placebo-controlled study.Gut. 2000; 47: 199-205Crossref PubMed Scopus (171) Google Scholar, 43Scolapio J.S. Effect of growth hormone, glutamine, and diet on body composition in short bowel syndrome a randomized, controlled trial.Jpen. 1999; 23: 309-312Crossref PubMed Scopus (88) Google Scholar A recent study reported by Seguy et al showed that low-dose growth hormone (0.05 mg/kg/day) was associated with a modest increase in carbohydrate and nitrogen absorption.44Seguy D. Vahedi K. Kapel N. et al.Low-dose growth hormone in adult home parenteral nutrition-dependent short bowel syndrome patients a positive study.Gastroenterology. 2003; 14: 293-302Abstract Full Text PDF Scopus (186) Google Scholar A recent randomized, placebo (glutamine)-controlled study indicated growth hormone administration, when used outside the natural adaptation period, led to the ability to reduce parenteral fluid and nutrition requirements, although absorptive studies were not undertaken.45Byrne T.A. Wilmore D.W. Iyer K. Dibase J. Clancy K. Robinson M.K. Chang P. Gertner J.M. Lautz D. Growth hormone, glutamine, and an optimal diet reduces parenteral nutrition in patients with short bowel syndrome a prospective, randomized, placebo-controlled, double-blind clinical trial.Ann Surg. 2005; 242: 655-661Crossref PubMed Scopus (166) Google Scholar Neither growth hormone nor glutamine have been studied in humans when used in the initial 1–2-year periadaptative period. Therefore, treatment with glutamine and/or growth hormone cannot be recommended at the present time. However, it may be that use of such exogenous growth factors may have their greatest utility if used during the normal adaptive period. There are no human studies to test this hypothesis. Lipid digestion may be impaired because of impaired solubility because micelle formation will be impaired when ileal bile salt malabsorption occurs in the setting of ileal resection (>100 cm).46Ohkohchi N. Andoh T. Izumi U. et al.Disorder of bile acid metabolism in children with short bowel syndrome.J Gastroenterol. 1997; 32: 472-479Crossref PubMed Scopus (31) Google Scholar Treatment with ox bile supplements has been attempted in 3 patients in an attempt to increase duodenal bile salt concentration to stimulate micellar lipid solubilization.47Little K.H. Schiller L.R. Bilhartz L.E. Fortran J.S. Treatment of severe steatorrhea with ox bile in an ileectomy patient with residual colon.Dig Dis Sci. 1992; 37: 929-933Crossref PubMed Scopus (18) Google Scholar, 48Fordtran J.S. Bunch F. Davis G.R. Ox bile treatment of severe steatorrhea in an ileectomy-ileostomy patient.Gastroenterology. 1982; 82: 564-567PubMed Scopus (21) Google Scholar, 49Djurdjevic D. Popovic O. Necic D. Hranisavljevic S. Ox bile treatment of severe steatorrhea in a colectomy and ileectomy patient.Gastroenterology. 1988; 95 (letter): 1160PubMed Google Scholar This therapy has been associated with significantly increased fecal volume, at least in those patients with intact colon. A preliminary, open-labeled study of 4 patients (2 with colon in continuity) indicated that treatment with the conjugated bile acid cholylsarcosine (6 g/day) was associated with an increase in fat absorption of 17 ± 3 g/day without any effect on stool wet weight.50Heydorn S. Jeppesen P.B. Mortensen P.B. Bile acid replacement therapy with cholylsarcosine for short-bowel syndrome.Scand J Gastroenterol. 1999; 34: 818-823Crossref PubMed Scopus (49) Google Scholar Cholylsarcosine is a conjugated bile acid and is resistant to colonic bacterial deconjugation. Another case series of 4 subjects had similar findings.51Kapral C. Wewalka F. Praxmarar V. Lenz K. Hofmann A.F. Conjugated bile acid replacement therapy in short bowel syndrome patients with residual colon.Z Gastroenterol. 2004; 42: 583-588Crossref PubMed Scopus (29) Google Scholar However, 1 of the 4 patients experienced a significant increase in wet stool output, and nausea developed in another patient. Cholestyramine should not be used in patients with >100 cm of ileal resection because it may actually worsen steatorrhea because of the binding of dietary lipid.52Hoffman A.F. Poley R. Role of bile acid malabsorption in pathogenesis of diarrhea and steatorrhea in patients with ileal resection.Gastroenterology. 1972; 62: 918-934PubMed Google Scholar Regardless of whether a high- or low-fat diet is used, the percentage of fat absorption remains stable, and stool weight is unaffected by the fat content of meals.53Ovesen L. Chu R. Howard L. The influence of dietary fat on jejunostomy output in patients with severe short bowel syndrome.Am J Clin Nutr. 1983; 38: 270-277PubMed Google Scholar, 54Woolf G.M. Miller C. Kurian R. Jeejeebhoy K.N. Diet for patients with a short bowel high fat or high carbohydrate?.Gastroenterology. 1983; 84: 823-828PubMed Google Scholar However, because of the energy density of fat (9.0 kcal/g) when compared with carbohydrate (4.0 kcal/g), it is an important dietary energy source. In addition, in short bowel syndrome patients, up to 65% of dietary carbohydrate may be malabsorbed and lost in the feces without degradation by colonic bacteria.32Ameen V.Z. Powell G.K. Jones L.A. Qualitation of fecal carbohydrate excretion in patients with short bowel syndrome.Gastroenterology. 1987; 92: 493-500PubMed Google Scholar A mixed long-chain triglyceride:medium-chain triglyceride (LCT:MCT) diet was studied in 19 patients. Those patients with intact colon absorbed 96% ± 3% of C8 fatty acids and 87% ± 6% of C10 fatty acids, and those patients without colon absorbed 63% ± 25% of C8 and 57% ± 28% of C10, (P = .007 for C8 and P = .004 for C10).55Jeppesen P.B. Mortensen P.B. The influence of a preserved colon on the absorption of medium chain fat in patients with small bowel resection.Gut. 1998; 43: 478-483Crossref PubMed Scopus (157) Google Scholar MCT contains 8.3 kcal/g. Energy absorption (approximately 2.1 MJ/day; 500 kcal/day) was significantly increased in patients with colon, although the LCT:MCT diet did not result in increased energy absorption when compared with the LCT-based diet in patients with an end-jejunostomy or ileostomy. These patient also had increased fecal output with a mixed LCT:MCT diet. Some, but not all LCT, can be replaced by MCT in the diet because the essential fatty acid linoleic acid is a constituent of LCT and is not found in MCT. Excessive MCT intake may result in nausea, vomiting, and ketosis. In a short bowel patient eating 10.5 MJ/day (2